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ARACHNODACTYLY (MARFAN'S SYNDROME) WITH SEVERE ANEMIA

ARACHNODACTYLY (MARFAN'S SYNDROME) WITH SEVERE ANEMIA • The condition characterized by elongation and narrowness of the long bones, particularly those of the metacarpals, fingers and toes, was named dolichostenomélie (long narrow limbs)) by Marfan in 1896.1 It is now more generally called arachnodactyly (spider fingers), a term first used by Achard in 1902.2 About 200 cases have been reported to date.3 Several observers4 have found a definite familial incidence in arachnodactyly. The condition is important chiefly because of serious lesions that accompany the bony abnormality. As is mentioned by Lloyd4a and Burch,4c dislocation of the lenses of the eyes occurs in at least half of the cases. Spinal curvature was reported first by Méry and Babonneix5 and is usually present,4a being found especially as kyphosclerosis. These spinal deformities are generally attributed to muscular weakness rather than to bony deformity. Piper and Irvine-Jones6 stated that congenital disease of http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American journal of diseases of children American Medical Association

ARACHNODACTYLY (MARFAN'S SYNDROME) WITH SEVERE ANEMIA

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References (5)

Publisher
American Medical Association
Copyright
Copyright © 1940 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0096-8994
eISSN
1538-3628
DOI
10.1001/archpedi.1940.02000030192021
Publisher site
See Article on Publisher Site

Abstract

• The condition characterized by elongation and narrowness of the long bones, particularly those of the metacarpals, fingers and toes, was named dolichostenomélie (long narrow limbs)) by Marfan in 1896.1 It is now more generally called arachnodactyly (spider fingers), a term first used by Achard in 1902.2 About 200 cases have been reported to date.3 Several observers4 have found a definite familial incidence in arachnodactyly. The condition is important chiefly because of serious lesions that accompany the bony abnormality. As is mentioned by Lloyd4a and Burch,4c dislocation of the lenses of the eyes occurs in at least half of the cases. Spinal curvature was reported first by Méry and Babonneix5 and is usually present,4a being found especially as kyphosclerosis. These spinal deformities are generally attributed to muscular weakness rather than to bony deformity. Piper and Irvine-Jones6 stated that congenital disease of

Journal

American journal of diseases of childrenAmerican Medical Association

Published: Sep 1, 1940

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